Kimura's disease: a case report.

BACKGROUND: Kimura's disease is a rare chronic inflammatory disorder of unknown etiology that is seen in people of Asian descent. It is characterized by head and neck subcutaneous nodules along with lymphadenopathy, which is usually solitary but can be generalized. It is diagnosed histopathologically by the proliferation of blood vessels and germinal centers in lymphoid follicles, along with variable degrees of fibrosis and extensive eosinophil infiltration. Its localized form is treated with surgical excision, while generalized lesions and those that do not respond to surgical excision can be managed with steroids or radiotherapy. CASE: In this report, we present the first case of Kimura's disease in the Ethiopian literature in a 40-year-old Ethiopian man that presented with generalized pruritic subcutaneous nodules and lymphadenopathy, which were effectively managed with a tapering course of prednisolone, and a relapse that showed good sustained response with slow steroid taper. CONCLUSION: We have demonstrated that, even though it is very rare in the African continent, Kimura's disease is to be considered as a differential diagnosis for patients that present with subcutaneous nodules and lymphadenopathy. We also have demonstrated that relapses can be effectively managed with reinitiation of the same dose of steroids but with a very slow taper.

Successful Management of Refractory Kimura Disease with CVP Chemotherapy: A Case Report.

BACKGROUND Kimura disease is a rare, chronic inflammatory disorder typically presenting as a painless mass in the head or neck and associated with elevated serum immunoglobulin E and blood and tissue eosinophilia. Generally benign, its management is not well-defined, but corticosteroids are a common initial treatment. We detail a case of refractory Kimura disease successfully managed with CVP (Cyclophosphamide, Vincristine, Prednisone) chemotherapy and no recurrence during 6 rounds of treatment. CASE REPORT A 64-year-old woman, previously diagnosed with Kimura disease, returned to the hospital with upper eyelid ptosis. Upon examination, a solid mass was palpable in her left upper eyelid. Peripheral blood tests confirmed elevated IgE levels at 356.0 IU/ml. An excisional biopsy showed infiltration of lymphocytes and eosinophils, consistent with Kimura disease. Despite undergoing corticosteroid treatment, surgical debulking, radiation, and immunosuppressant therapy, her condition worsened. Concerns were raised due to imaging features suggestive of lymphoma, although no malignancy was evident in subsequent biopsies. It was decided to manage the disease using CVP chemotherapy, leading to significant symptom improvement. There have been no recurrences during the 12-month follow-up period. CONCLUSIONS Kimura disease is typically benign and responsive to treatment, but it often recurs and can progress. When symptoms are not controlled with conventional treatments, including corticosteroids, immunosuppressants, radiation, and surgical debulking, chemotherapy may be a reasonable option even when no definite signs of malignancy is identified. Further research is needed to explore the utility of CHOP and CVP in managing uncontrolled Kimura disease.

Kimura's disease as a mimicker of cavernous hemangioma: A case report and literature review.

Kimura's disease (KD) is an immune-mediated disorder which mainly affects Asian men. It appears as head and neck subcutaneous masses, with inflammatory infiltrate and elevated serum immunoglobulin E levels. The clinical presentation of KD resembles that of various diseases. Here, we report the case of a 30-year-old Filipino man with KD mimicking cavernous hemangioma who was treated by surgery. Careful survey for possible KD cases is crucial. Misdiagnoses are prone to futile interventions and unwanted effects. Surgery with adjuvant therapy is superior to other forms of KD treatment.

Anti-immunoglobulin E provides an additional therapy to conventional steroids for Kimura's disease.

Kimura's disease (KD) is a chronic inflammatory disease characterized by painless subcutaneous head and neck swelling, eosinophilia, and elevated serum immunoglobulin (Ig) E levels. There are various therapies, including surgery, radiation, systemic steroids, and immune suppressants, but their efficacy remains moderate due to the high recurrence rate. Biologics, like monoclonal antibodies, have shown tremendous effectiveness for chronic inflammatory diseases. Omalizumab is a monoclonal antibody against IgE and has not been approved for KD so far. We describe two refractory KD cases that responded to a small dose of steroids plus omalizumab. Additionally, we reviewed another 13 KD cases that were treated with biologics, including omalizumab, rituximab, dupilumab, and mepolizumab. The results indicate that biologics provide an alternative treatment strategy for KD.

Diagnostic challenges and updated therapeutic strategies of Kimura's disease: A case report successfully treated by dupilumab and review.

RATIONALE: Kimura's disease (KD) is a rare and chronic eosinophilic related-disease, characterized by subcutaneous tissue masses, regional enlarged lymph nodes, hypereosinophilia and elevated serum IgE. KD usually affects young adults in the Asian population. In Western countries, the clinical and biological presentation of KD is often unknown, delaying the diagnosis. Therapeutic management is not standardized and despite recent advances, remission from KD can be difficult to achieve, especially in relapse situations. PATIENT CONCERNS: We report the case of an non-Asian man with KD, initially misdiagnosed as lymphoma. We focus on his long-lasting clinical course with 20 years of recurrence despite several therapeutic lines. DIAGNOSES AND INTERVENTIONS: We have emphasized the key points of the KD diagnostic challenge. We chose to focus on hemopathies as diagnostic traps to illustrate several overlapping features that blur frontiers with KD. With regard to treatments, lessons can be learned from the use of the therapeutic backbone, which relies on excision surgery, radiotherapy and corticosteroids. OUTCOMES: Advancements in KD pathogenesis have highlighted the pivotal role of Th2 lymphocytes driving eosinophil activation. Directly inspired by eosinophilic and allergic field practices, targeted therapies, such as dupilumab, provide hope for potential curative options. LESSONS: Finally, we propose a therapeutic plan to treat newly diagnosed KD and discuss options for relapsing entities.

Clinical effects of dupilumab: A novel treatment for Kimura disease.

BACKGROUND: Kimura disease (KD) is a rare chronic inflammatory disorder involving the Th2 pathway. Although medical treatment with steroids or other immunosuppressants is available, they may cause developmental issues in the pediatric population. Surgical intervention has also been suggested; however, it is associated with high recurrence rates. CASE PRESENTATION: A 14-year-old boy presented with left retroauricular lymph node enlargement at the age of 5 years. At the age of 7 years, he was diagnosed with nephrotic syndrome which subsided after steroid treatment for approximately 6 years. The retroauricular lymph node was surgically excised, and KD was confirmed. However, recurrent enlargement of the left retroauricular and neck lymph nodes occurred after 2 years. Persistently high IgE levels and fluctuating eosinophil counts were observed following steroid treatment. Dupilumab was prescribed because of the difficulty in tapering the steroid dosage. A loading dose of 600 mg was administered, followed by a maintenance dose of 300 mg every 2 weeks. The IgE level decreased after 3 months, and a low eosinophil count was maintained after steroid discontinuation. Follow-up computed tomography revealed a decrease in the size of the lymph nodes with no side effects such as conjunctivitis. CONCLUSION: Traditional treatments have raised developmental concerns in the pediatric population and are associated with high recurrence rates. Dupilumab targets the Th2 pathway and provides effective results, with few adverse effects. Dupilumab may be a therapeutic option for KD and other diseases involving the Th2 pathway.

Combination of available topical beta-blockers and antibiotic ointment for epidermal growth factor receptor tyrosine kinase inhibitor-induced paronychia and pseudopyogenic granulomas in Taiwan.

BACKGROUND: Painful paronychia and pseudopyogenic granuloma (PG) are common adverse drug reactions (ADRs) associated with the use of epidermal growth factor receptor tyrosine kinase inhibitors (EGFR-TKIs) to treat non-small cell lung cancer (NSCLC). Multiple local management approaches have been tested with unsatisfactory results. We have introduced an occlusion therapy technique through which available topical drugs for longer than 2 years. METHODS: Based on the cancer registry and case management system of our hospital, from July 2019 to July 2020, we retrospectively enrolled patients with NSCLC who were treated with EGFR-TKIs and received applications of 0.5% timolol ophthalmic solution (TIMOPTOL XE 0.5%®) combined with a neomycin/tyrothricin ointment (Biomycin®) using the occlusion method to treat paronychia or PG. RESULTS: A total of 22 patients were enrolled, with a mean age of 66.5 years, most of whom were women (72.7%). Periungual lesion-related pain was reported by all patients, and periungual bleeding and PG were reported in 14% (3/22) and 64% (14/22) of patients, respectively. After the occlusion therapy application of timolol ophthalmic solution combined with neomycin/tyrothricin ointment twice daily, the overall response rate was 83.3%, including complete response in 18% (4/22) of cases and partial response in 68% (15/22) of cases. CONCLUSION: We presented an occlusion method using available topical beta-blockers and antibiotic ointment for EGFR-TKI-induced paronychia and PG in Taiwan. The result is favorable. Further randomized control trial is urgent to validate our findings.

[Kimura's disease. (Clinical case study)]. / Bolezn' Kimury. (Sobstvennoe nablyudenie).

The author presents his own observation of a patient with Kimura's disease (CD), which is a rather rare chronic inflammatory disease of soft tissues. The results of clinical examination and surgical treatment of this disease are presented. It is emphasized that for the diagnosis of CD, first of all, an excision biopsy of the lesion is necessary, as well as the presence of eosinophilia in the patient, an increased level of immunoglobulin E (IgE) in peripheral blood, an increase in regional lymph nodes.

Refractory angiolymphoid hyperplasia with eosinophilia: Complete resolution with low dose thalidomide.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a difficult-to-treat and rare benign vascular proliferative condition which presents as painless, solitary, or multiple vascular papules or nodules in the head and neck area, with predilection for the ear. We report a case of ALHE that failed multiple therapeutic interventions but achieved complete resolution when treated with thalidomide.

A retrospective study of 20 patients with Kimura's disease from China.

BACKGROUND: Kimura's disease (KD) is an eosinophilic proliferative lymphoid disease with rare incidence rate and nonspecific clinical symptoms. OBJECTIVES: To investigate the clinical characteristics, diagnosis, treatment, and prognosis of KD. MATERIALS AND METHODS: The clinical manifestations, laboratory examination, imaging examination, proposed treatment, and prognosis of 20 patients with KD treated in our hospital were retrospectively analyzed. RESULTS: All cases showed painless masses. The proportion of peripheral blood eosinophils rose in 18 cases (90%), the eosinophil count increased in 14 cases (70%) and the serum immunoglobulin E (IgE) levels were significantly increased in 5 patients (100%). Pathological examination revealed follicular hyperplasia, eosinophils infiltration, and tiny vessels proliferation in KD samples. CONCLUSIONS: KD should be considered when the patients suffered head and neck subcutaneous painless mass, increased peripheral blood eosinophils and raised serum IgE level. Pathological examination is the golden standard for diagnosing KD. Surgical intervention, medical treatments, and radiotherapy may be effective for KD treatment. SIGNIFICANCE: This study might shed a light on improving the diagnosis and treatment for KD.

Kimura's disease: clinical characteristics, management and outcome of 20 cases from China.

OBJECTIVES: To explore the clinical characteristics, diagnosis and the therapeutic effect of Kimura's disease (KD). METHODS: Clinical data of 20 patients with pathologically confirmed KD admitted to Peking University People's Hospital from June 2000 to June 2019 were analysed. A total of 20 confirmed KD patients were enrolled in the study, 18 male and 2 female, with age-onset ranging from 2 to 58 years. RESULTS: The masses appear as focal, painless, and immovable with an unclear boundary. The most common predilection is head-neck region (n=15, 75%). 15 patients showed peripheral blood eosinophilia. 14 of 14 patients presented with increased serum IgE level. The prominent pathological characteristic is marked lymphoid hyperplasia accompanied by various degrees of vascular hyperplasia and eosinophil infiltration. Among the 20 patients, 12 experienced recurrence of disease after treatment (surgical resection alone: 9/9; oral corticosteroids combined with immunosuppressants: 1/3; surgical resection followed by oral corticosteroids combined with immunosuppressants: 2/6). CONCLUSIONS: KD should be considered when the patient presents with head-neck swellings and lymphadenopathy, accompanied by an increase of IgE and eosinophil. Compared with surgery alone, combined therapy seems to be a promising treatment option to reduce the recurrence rate.

A case of membranous nephropathy with Kimura's disease.

BACKGROUND: The association between membranous nephropathy (MN) and Kimura's disease (KD) has been reported in recent years. The treatment, effect, and prognosis of KD are still unclear. CASE REPORT: A 47-year-old KD patient developed a left axillary mass for 3 years and received surgical resection because of the lager mass in August 2016. Then he developed nephrotic syndrome 3 months later. Laboratory index revealed increased eosinophil count, decreased albumin and heavy proteinuria. Lymph node biopsy suggested KD, and renal biopsy suggested MN. He relapsed after a treatment with methylprednisolone (52 mg/d) alone and then tacrolimus (1.5 mg/12h) was added. The patient had no symptoms of relapse in the next 2 years. CONCLUSION: The combination therapy of surgery, methylprednisolone, and immunosuppressive agents may be effective in KD with MN.

Recurrent Kimura's disease of head and neck treated with intensity-modulated radiotherapy.

Kimura's disease (KD) is a rare, chronic inflammatory disorder of unknown aetiology, which commonly affects men of the Asian race. Here, we present a case capsule of a 39- year-old man with KD of the left cheek, managed initially by surgery alone. He developed local recurrence after 6 months and was treated with steroids and isotretinoin. Eventually, steroids were discontinued due to toxicity and the lesion progressively increased in size. The patient was successfully treated using intensity-modulated radiotherapy with simultaneous integrated boost as a primary modality with minimal adverse effects. The patient has good local control and cosmetic outcome with no radiation-related toxicity at a follow-up period of 28 months.

[Immune thrombocytopenia associated with Kimura's disease].

In this study, we report a rare case of immune thrombocytopenic purpura (ITP) associated with the worsening of Kimura's disease. A 47-year-old Japanese man with a pruritic rash and swollen inguinal lymph nodes was diagnosed with Kimura's disease on performing a right inguinal lymph node biopsy. Thrombocytopenia ensued after the diagnosis of Kimura's disease, and fluctuations in the platelet count were observed along with the pathology of Kimura's disease. The platelet count fluctuated repeatedly with the relapse of Kimura's disease and a diagnosis of a combination of Kimura's disease and ITP was made through lymph node regeneration and bone marrow examination. Treatment with prednisolone (1 mg/kg/day) was initiated for Kimura's disease and ITP, and lymphadenopathy and platelet count improved promptly. Since then, the dose of prednisolone has been gradually reduced, and the disease status of both Kimura's disease and ITP has been controlled.

Concurrent Kidney Glomerular and Interstitial Lesions Associated with Kimura's Disease.

BACKGROUND: Kimura disease (KD) is a chronic benign inflammatory disorder that is usually manifested as a deep, subcutaneous mass with or without regional lymphadenopathy in the head and neck region. Various types of glomerulonephritis are associated with KD, including minimal change disease (MCD), membranous glomerulopathy (MN), and immunoglobulin (Ig)A-nephropathy. Kidney interstitial lesion associated with KD is rarely reported. The aim of this study was to expand the spectrum of kidney lesions associated with KD. METHODS: From 2007 to 2016, 12 cases of KD with kidney lesions were retrospectively reviewed. Pathological examinations included hematoxylin and eosin staining, periodic acid-schiff staining, periodic acid-methenamine silver staining, and Masson staining, immunofluorescence, and electron microscope analyses. RESULTS: Anatomic sites of subcutaneous involvement included head and neck area, arm, and groin. Most cases had elevated IgE level and peripheral eosinophilia. Nephrotic syndrome was the most common kidney manifestation. Pathological results showed 3 types of glomerulonephritis, including 9 cases of MCD, 2 cases of IgA nephropathy, and 1 case of MN. Of note, 4 MCD cases showed concurrent glomerular and interstitial lesions associated with KD, including 2 distinct patterns: (1) a diffusely eosinophilic and lymphatic infiltration similar to KD lesion developed elsewhere and (2) exclusively eosinophilic infiltration in the interstitium. Eight out of 10 patients were responsive to steroid treatment and had complete remission of proteinuria and recovery of kidney function. CONCLUSION: Our report suggested that, in addition to glomerulonephritis, interstitial lesions may also be associated with KD. Pathologist should pay special attention to differentiated diagnosis when such pathological changes are identified in patients with subcutaneous mass or cervical lymphadenopathy with concurrent kidney manifestation.